Hageman factor (HF) deficiency

A patient with Hageman factor (HF) deficiency is described. This syndromeis characterized by complete absence of any hemorrhagic tendency in the presence of laboratory findings which, as a rule, are associated with severe disturbances in the hemostatic mechanism. The clotting time was markedly prolonged,the plasma prothrombin time was normal, but prothrombin consumption wasdecreased. The thromboplastin generation test revealed that HF is essential forblood thromboplastin formation at least in vitro.

Procedures for the differentiation of HF deficiency from AHF, PTC and PTAdeficiency syndromes are outlined.

Transfusions of as little as 50 cc. of 20 day old blood normalized the abnormalclotting tests immediately for a period of about 36 hours.

The basis for the apparent lack of need for preoperative preparation withblood transfusions in HF deficiency is discussed.

Submitted on November 21, 1955 Accepted on February 6, 1956